Rheumatic FeverBy Dr. Saud A. Bahaidarah

Pediatric cardiologist

Definition

Delayed autoimmune reaction to group A, B-hemolytic streptococcal pharyngitis in genetically susceptible individuals .

Involves : heart, joints, brain, skin, serous

surfaces

Epidemiology Prevalence

developed countries , RF was decreasing science 1920’s and almost disappeared in 1980’s , and reappear again in 1985 due to reappearance of streptococcus with M-protein serotype.

In developing countries , RF is a major problem affecting about 10-20 million per year, RHD is a primary cause of death below 50 years of age

Incidence:

In developing countries : 150 per 100,000 with mortality rate up to 8.2 per 100,000 from cardiac involvement

In developed countries : less than 1 per 100,000 with mortality rate of 1.8 per 100,000

Environment :overcrowding, poverty, poor nutrition, poor

hygiene, and poor access to health care are common in developing countries and contribute to rapid spread (respiratory droplets) and increased virulence of GAS .

With poor access to health care, GAS pharyngitis is less likely to be diagnosed and treated, precluding effective primary prevention of RF

Both GAS pharyngitis and RF are more common during the winter and spring in temperate climates, but there is no consistent seasonal pattern in the tropics

Host :Age : 5-15 years of age with peak at 8 yearsUncommon before 5 years and after 35 years

of age Almost never before 2 years of life No gender predisposition except for chorea in

girlsRecurrence in adolescent and early adulthood

Family history indicates genetic factor which still under study

Pathogenesis Organism : group A, B hemolytic

streptococcusSerotype : depends on M-protein :

Class I : infect pharynx causing RFClass II : infect skin causing

glomerulonephritis Latent period : 10 days to 5 wk, average of 18

daysInfection lead to activation of T-cells and B-

cell and release of cytokines and antibodies which will attack the myosin the heart .

Pathology Aschoff nodules :

perivascular aggregation characterized by a central area of fibrinoid change (altered collagen) surrounded by or infiltrated by large multinucleated cells

Found in all affected organs i.e. heart, skin, brain, joint, serous surfaces.

Clinical picture

History of sore throat about 3wk back

History of fever with other major or minor manifestation

ArthritisCommon in 70% of RF casesMigratory, non destructive arthritis Involves large joints with all signs of

inflammation mostly knees ,hips, ankles, elbows, wrist, shoulder

Duration not more than 2-3 days Self limited Respond dramatically to salisylate

Carditis About 50% of RF will be affected Ranges from asymptomatic , heart murmur to

sever heart failure Usually it is pancarditis Affecting mitral valve 95% , aortic valve

25% , tricuspid valve and rarely pulmonary valve

Pericarditis in 4-10%

Sydenham's chorea In 10-30 % of patient with RFDue to inflammation in the basal ganglia, cerebral cortex,

and the cerebellum Involuntary, purposeless movements, muscular

incoordination and/or weakness, and emotional lability tongue movements described as resembling a bag of wormsExplosive speech pronation of the hands when arms are extended above the

head (pronator sign), irregular contractions of the hands when asked to squeeze

an object (milkmaid's grip) hyperextension of the fingers when hands are extended

forward with eyes closed, (spooning)

Patients often come to attention based on deterioration in school performance, and neurobehavioral symptoms seen along with the chorea including irritability, poor attention span, lack of cooperation, and obsessive-compulsive symptoms are not uncommon.

Sensory deficits do not occur. The neurologic manifestations are usually bilateral

but may be unilateral (hemichorea). These symptoms, which decrease with rest and

sedation and increase with effort or excitementResolve over a median of 15 weeks, and by 6

months in 75% of cases . Recurrent episodes of chorea are not uncommon

Latent period in chorea is about 1-6 month while in arthritis 10-18 days , this can explain that both don’t present together .

If chorea and cardiac involvement together most likely the cardiac involvement is mild with decreased acute phase reactant

Erythema Marginatum occurs in less than 10% of patients with acute

rheumatic fever. The characteristic nonpruritic serpiginous or

annular erythematous rashes are most prominent on the trunk and the inner proximal portions of the extremities; they are never seen on the face.

The rashes are evanescent, disappearing on exposure to cold and reappearing after a hot shower or when the patient is covered with a warm blanket.

They are seldom detected in air-conditioned rooms.

Subcutaneous nodules Found in 2% to 10% of patients, particularly in

cases with recurrences They are hard, painless, nonpruritic, freely

movable, swelling, and 0.2 to 2 cm in diameter. They are usually found symmetrically, singly or

in clusters, on the extensor surfaces of both large and small joints, over the scalp, or along the spine.

They are not transient, lasting for weeks Have a significant association with carditis.

Laboratory :Increase in acute phase reactant Evidence of streptococcal infection :

Positive throat swap Strep antibodies :

Antistreptolysin O (ASO) titer is well standardized and therefore is the most widely used test. It is elevated in 80% of patients with acute rheumatic fever and in 20% of normal

individuals. Only 67% of patients with isolated chorea have an elevated ASO.

Antideoxyribonuclease B titer The Streptozyme test

ECG :Prolongation of PR interval Occasional 2:1 block, rarely complete heart

blockNonspesific T wave and ST segment changes

CXR:Enlareged heart Evidence of increase PVM if CHF

Echocardiography :Evaluate the effect of carditis on the heart

including MR, AR, MS, preicardial effusion, and function

Diagnosis

Moss and Adam’s Heart Disease in Infant, Children, and Adolescents 7th edition

Treatment Includes :

Bed rest

Anti-inflammatory

Heart failure managements

Antibiotics : primary and secondary prevention

Bed rest

Pediatric Cardiology for practitioners 4th edition

Anti-inflammatory

Mild to moderate carditis: aspirin 80-100 mg/kg/day in 4 divided doses for children 4-8 g/day in adolescents and adults Target salicylate levels 20-30 mg/dL Don't forget to protect the stomach

Severe carditis: Initial steroids (prednisone 2 mg/kg/day) for

approximately 2 weeks, then taper Begin aspirin approximately 1 week prior to

stopping steroids to prevent rebound Follow acute phase reactants (erythrocyte

sedimentation rate, C-reactive protein)

Heart failure managements

Depending on the severity Salt and water restrictionDiuretics Afterload reduction Surgery in sever cases that needs

intervention and refractory to medical management

Antibiotics : primary prevention To eradicate the

organism Doesn't interfere

with disease course

Moss and Adam’s Heart Disease in Infant, Children, and Adolescents 7th edition

Antibiotics : secondary preventionTo prevent the

recurrence and then worsening of the RHD

Recurrence rate 40-60 %

Higher in early years after 1st attack then decreases with time

Moss and Adam’s Heart Disease in Infant, Children, and Adolescents 7th edition

Thank You